Hereditary deficiency of vitamin-K-dependent coagulation factors in Rambouillet sheep

A flock of Rambouillet sheep experienced unexpected lamb mortality associated with excessive bleeding at the time of parturition. Most lambs died of blood loss through the umbilicus or into subcutaneous tissues. Subsequently, nine ewes which had previously delivered lambs that bled to death were bre...

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Bibliographic Details
Published in:	Blood coagulation & fibrinolysis Vol. 10; no. 2; p. 75
Main Authors:	Baker, D C, Robbe, S L, Jacobson, L, Manco-Johnson, M J, Holler, L, Lefkowitz, J
Format:	Journal Article
Language:	English
Published:	England 01-03-1999
Subjects:	Animals Blood Coagulation Factors - analysis Blood Coagulation Factors - immunology Coagulation Protein Disorders - blood Coagulation Protein Disorders - genetics Coagulation Protein Disorders - veterinary Counterimmunoelectrophoresis Factor VII - analysis Factor X Deficiency - blood Factor X Deficiency - genetics Factor X Deficiency - veterinary Female Genes, Recessive Hemophilia B - genetics Hemophilia B - veterinary Liver - chemistry Male Partial Thromboplastin Time Protein C - analysis Protein C - immunology Protein S - analysis Protein S - immunology Prothrombin - analysis Sheep Sheep Diseases - blood Sheep Diseases - genetics Thrombin Time Vitamin K - antagonists & inhibitors Vitamin K - metabolism
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Summary:	A flock of Rambouillet sheep experienced unexpected lamb mortality associated with excessive bleeding at the time of parturition. Most lambs died of blood loss through the umbilicus or into subcutaneous tissues. Subsequently, nine ewes which had previously delivered lambs that bled to death were bred to the suspected sire of the previous bleeding lambs. Fifteen lambs were born alive the following Spring, and three males and one female bled clinically. These lambs had markedly decreased factor IX (< 16%) and factor X (< 4%) activities, with variably decreased factor II (11-36%) and factor VII (20-37%) activities. Protein C chromogenic activity was also markedly decreased (< 1%) in these lambs. The results from crossed immunoelectrophoresis and 'protein-induced-in-vitamin-K-absence' determination of the plasma of affected lambs, with antiserum directed against coagulation factor X, protein C or proteins S, suggested that these proteins were not carboxylated normally. Examination of liver from one lamb in the first batch and the four subsequent lambs did not reveal a known vitamin K antagonist. The breeding data suggested that the coagulopathy in these sheep was inherited as an autosomal recessive trait. The genetic or molecular defect that exists in these lambs is unknown, but possibilities include abnormal gamma-glutamyl carboxylase activity or abnormal metabolism of vitamin K.
ISSN:	0957-5235
DOI:	10.1097/00001721-199903000-00003